老年肾脏原始神经外胚层肿瘤——病例分析并文献复习

K. Kavitha, G. Kottangal, Shehla Basheer Kollathodi, Shalini Kuruvila, L. Madhavan, Surdas Radhakrishnan
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摘要

摘要原发性肾神经外胚层肿瘤是一种非常罕见且高度侵袭性的肿瘤,常见于青少年。这些肿瘤在40岁以上的患者中很少见。在60岁以上的老年患者中出现肾PNET的报道更为罕见。我们报告三例肾PNET在成人患者诊断超过三年的时间。3例患者中2例年龄在60岁以上,第3例患者为39岁男性。所有三例患者在就诊时均有广泛的静脉血栓形成。1例患者术后立即死亡,另外2例接受化疗。肾脏PNET因其在中老年少见,对病理学家的诊断提出了挑战。临床和放射学表现对准确诊断有局限性。彻底的形态学评估以及免疫组织化学和细胞遗传学分析有助于达到正确的诊断。准确的诊断对于开始适当的治疗至关重要。
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Primitive neuroectodermal tumor of kidney in elderly -A case series with review of literature
Primitive neuroectodermal tumor of the kidney (PNET) is a very rare and highly aggressive tumor commonly seen in young adolescents. These tumors are rare in patients over 40 years. Reports of renal PNET in elderly patients over 60 years are even rarer.We report three cases of renal PNET in adult patients diagnosed over a period of three years. Among the three patients, two were above 60 years of age and the third patient was a 39-year-old male. All the three patients had extensive venous thrombosis at the time of presentation. One of the patients succumbed to death in the immediate post-operative period and the other two received chemotherapy.Renal PNET owing to its rare occurrence in middle-aged and elderly poses a diagnostic challenge to the pathologist. The clinical and radiological findings have limitations in the accurate diagnosis. Thorough morphological evaluation along with immunohistochemistry and cytogenetic analysis help in arriving at a correct diagnosis. An accurate diagnosis is crucial for the initiation of proper treatment.
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